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F1000Research 2019Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a... (Review)
Review
Pyoderma Gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. PG has historically been a challenging disorder to diagnose, leading to the development of new diagnostic criteria rather than the traditional approach of a diagnosis of exclusion. The pathophysiology is thought to involve both innate and adaptive immune system dysregulation, neutrophilic abnormalities, environmental, and genetic factors. As of today, no gold standard therapy exists for the treatment of PG, and the literature is restricted to mainly case reports, case series, and 2 small randomized clinical trials. Topical, systemic, and biologic therapy, as well as adequate analgesia and proper wound care all play a role in the management of PG. Recent studies have identified additional cytokines and signalling cascades thought to be involved in the pathogenesis of PG, ultimately leading to the development of new targeted therapies. This review will focus on recent advances in the pathophysiology, clinical presentation and associated comorbidities, diagnosis, and management of PG.
Topics: Cytokines; Humans; Pyoderma Gangrenosum
PubMed: 31885859
DOI: 10.12688/f1000research.19909.1 -
International Wound Journal Apr 2019Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to... (Review)
Review
Pyoderma gangrenosum (PG) is a rare ulcerative skin disease that presents a therapeutic challenge. Tumour necrosis factor alpha (TNFα) inhibitors have been reported to successfully control PG. Our aim was to systematically evaluate and compare the clinical effectiveness of TNFα inhibitors in adults with PG. A literature search including databases such as PubMed, Embase, Scopus, and Web of Science was conducted, using search terms related to PG and TNFα inhibitors. Studies and case reports were included if patients were diagnosed with PG, over the age of 18 and administered TNFα inhibitor. A total of 3212 unique citations were identified resulting in 222 articles describing 356 patients being included in our study. The study we report found an 87% (95% CI: 83%-90%) response rate and a 67% (95% CI: 62%-72%) complete response rate to TNFα inhibitors. No statistically significant differences in the response rates (P = 0.6159) or complete response rates (P = 0.0773) to infliximab, adalimumab, and etanercept were found. In our study TNFα inhibitors demonstrated significant effectiveness with response and complete response rates supporting the use of TNFα inhibitors to treat PG in adults. Our study suggests that there is no significant difference in effectiveness among infliximab, adalimumab, and etanercept.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Antirheumatic Agents; Etanercept; Female; Humans; Immunologic Factors; Infliximab; Male; Middle Aged; Pyoderma Gangrenosum; Tumor Necrosis Factor-alpha
PubMed: 30604927
DOI: 10.1111/iwj.13067 -
Head and Neck Pathology Dec 2017Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The... (Review)
Review
Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. The condition can affect any anatomical site, however the head and neck are rarely involved. Although the oral cavity is subject to recurrent minor trauma through everyday activities such as mastication and oral hygiene, as well as during dental treatment, oral lesions appear to be extremely rare. In an effort to provide a detailed explanation of the oral manifestations of PG, a systematic search was conducted using medical databases. A total of 20 cases of PG with oral involvement were reported in the English and French literature. The objectives of this article are to present the pertinent diagnostic criteria and to discuss the differential diagnosis and therapeutic modalities.
Topics: Diagnosis, Differential; Humans; Oral Ulcer; Pyoderma Gangrenosum
PubMed: 28275955
DOI: 10.1007/s12105-017-0804-3 -
BMJ Case Reports Oct 2010A 3.5-year-old girl presented with skin lesions beginning as a small pustule that progressed to very painful large ulcer with rolled up edges and irregular borders. At...
A 3.5-year-old girl presented with skin lesions beginning as a small pustule that progressed to very painful large ulcer with rolled up edges and irregular borders. At presentation, active ulcers were present over the left elbow. There were healed ulcers with scarring present on the chest, back, both thighs and abdominal wall. This child had negative antinuclear antibody, cytoplasmic antinuclear cytoplasmic antibody, antiphospholipid antibodies and tests for hepatitis B and C. She did not have any symptoms to suggest ulcerative colitis. Her colonoscopy was absolutely normal. Histopathology from her skin lesion was consistent with pyoderma gangrenosum.
Topics: Child, Preschool; Diagnosis, Differential; Female; Humans; Pyoderma Gangrenosum
PubMed: 22791727
DOI: 10.1136/bcr.04.2010.2942 -
Clinics in Dermatology 2023
Topics: Humans; Pyoderma Gangrenosum; COVID-19; Vaccination
PubMed: 36442730
DOI: 10.1016/j.clindermatol.2022.11.003 -
Journal of Ayub Medical College,... 2023The first description of Pyoderma gangrenosum (PG) was made about a century ago. It is difficult to understand the aetiology, pathophysiology, and therapy of PG. This... (Review)
Review
The first description of Pyoderma gangrenosum (PG) was made about a century ago. It is difficult to understand the aetiology, pathophysiology, and therapy of PG. This disease is believed to be caused by a systemic inflammatory response to neutrophil chemotaxis and faulty innate immune system control. Nearly fifty percent of the cases have underlying systemic symptoms. Significant improvements in PG management have been made over the years. The main goals of treatment are to reduce inflammation and speed up the healing of the PG wound. Even though the most recent medicines show promise, they are found on isolated case reports. The majority of patients are typically managed with topical treatment and local wound care, while resistant cases necessitate immunosuppressive medications. More progress can be made with improvements in technology in deciphering this complex disease and getting a greater understanding of the condition. The present standard therapies for refractory PG are not well supported by studies. In refractory PG, corticosteroids and cyclosporine have historically been administered. Tumour necrosis factor inhibitors are becoming a viable option; nonetheless, this requires careful research and upkeep. This review intended to describe the current trends in managing the PG. Several next-generation treatment options including the conventional therapies introduced to treat PG. We encompass the advantages and disadvantages of new treatments for PG.
Topics: Humans; Pyoderma Gangrenosum; Inflammation; Administration, Topical
PubMed: 38406909
DOI: 10.55519/JAMC-S4-12085 -
Deutsches Arzteblatt International Sep 2020
Topics: Aged; Biopsy; Chronic Disease; Humans; Male; Pyoderma Gangrenosum
PubMed: 33263537
DOI: 10.3238/arztebl.2020.0614a -
CMAJ : Canadian Medical Association... Sep 2019
Topics: Cyclosporine; Female; Humans; Immunosuppressive Agents; Leg; Lupus Erythematosus, Systemic; Middle Aged; Pyoderma Gangrenosum
PubMed: 31548193
DOI: 10.1503/cmaj.190721 -
Indian Journal of Dermatology,... 2023Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and... (Review)
Review
BACKGROUND
Pyoderma gangrenosum is a rare autoinflammatory neutrophilic dermatosis that rapidly evolves. However, little is known about the clinicopathological features and prognosis of pyoderma gangrenosum.
AIMS
We aimed to document clinicopathologic and prognostic data of the patients with pyoderma gangrenosum.
METHODS
In this retrospective observational study, we reviewed case records of patients diagnosed with pyoderma gangrenosum between 1999-2019.
RESULTS
Fifty-three patients were identified by reviewing medical records for skin biopsy; of these, 37 were men and 16 were women. Mean age at onset was 43.3 ± 18.5 years. The most frequently affected area was the lower extremities (60.4%), followed by the head and neck (17.0%). The most common subtype was ulcerative (47.2%), followed by bullous (22.6%). 30 cases had underlying diseases and the most common were malignancy (24.5%), followed by inflammatory bowel diseases (18.9%). The proportion of cases with history of trauma were significantly higher in post-operative type (100%) as compared to the bullous type (8.3%). Histologic features of granulation tissue were frequently found in post-operative type (66.7%) and bullous type (58.3%). Granulomas were predominantly found in bullous type (58.3%). Age <60 years appeared to be significantly associated with multiple lesions. Partial-to-complete remission was observed in 40 cases (75.5%). Nine (17.0%) cases experienced recurrence with a median progression-free period of six months (interquartile range of 3.0-9.0 months). Cases with underlying hematologic disorders and the bullous subtype were significantly associated with early recurrence.
LIMITATIONS
This study was a single-centre study with a retrospective design.
CONCLUSION
Pyoderma gangrenosum appears to have ethnic differences. Underlying haematologic disorders and bullous subtype have a worse prognosis. However, the type of histopathology did not correlate with the clinical outcome of pyoderma gangrenosum.
Topics: Male; Humans; Female; Young Adult; Adult; Middle Aged; Pyoderma Gangrenosum; Retrospective Studies; Inflammatory Bowel Diseases; Prognosis; Republic of Korea; Observational Studies as Topic
PubMed: 35389018
DOI: 10.25259/IJDVL_968_20 -
The Pan African Medical Journal 2020Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary... (Observational Study)
Observational Study
Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary study's aim was to examine all cases of PG observed in our department over a 6-year period in order to describe the relevant characteristics and outcome under therapy. Fourteen patients were included (5 women, 9 men). The average age of our patients was 40,15 years. The classical, ulcerative form was found in 10 cases (71.42%), the pustular form in 4 cases (27.57%) and PG was multifocal in 4 cases. The PG was located preferentially to the lower limbs. Histological examination was realized in all patients and objectified inflammatory infiltrate composed of polymorphonuclear neutrophils in all cases with vasculitis in 4 cases. Six patients (42.85%) had associated disease at diagnosis of PG, including inflammatory bowel disease in two cases (14.28%), a blood disease in 2 cases (14.28%), lymph node tuberculosis and inflammatory arthritis in 1 case (7%). The most frequent first-line treatments were oral corticosteroids (7 cases) and other treatments used were colchicine in 2 cases, topical corticosteroids in 3 cases with good clinical evolution. Our study confirms that PG is a rare disease, associated in almost half of cases with systemic disease already present at diagnosis; in our Moroccan background, it is most often inflammatory bowel disease, hematological or solid cancer and tuberculosis.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Child; Colchicine; Female; Humans; Male; Middle Aged; Prospective Studies; Pyoderma Gangrenosum; Treatment Outcome; Young Adult
PubMed: 32733629
DOI: 10.11604/pamj.2020.36.59.12329